POS1564 AUTOIMMUNE INNER EAR DISEASE WITH A RHEUMATOLOGIC APPROACH
Authors: M. Diaz Menindez, A. M. Chindris, R. Butendieck, Y. LI, C. Razvan, K. Britt, F. Berianu
Journal: Annals of the Rheumatic Diseases (2023)

<h3>Background</h3> Autoimmune inner ear disease (AIED) is a rare entity that presents with sensorineural hearing loss. In the US, the incidence rate is less than 5 per 100,000 people [1]. The pathophysiology behind the disease is thought to be a humoral and cell-mediated process that damages the cochlea [2]. Clinical presentation is characterized by fluctuating, asymmetric hearing loss that tends to progress over time. Half of patients with AIED present with vestibular (eg, dizziness, vertigo, imbalance) and other ear symptoms (eg, tinnitus, ear pressure and aural fullness) [2,3]. Diagnosis of AIED remains challenging as no diagnostic test has demonstrated high sensitivity or specificity [1]. Diagnosis remains clinical and based on response to corticosteroids, which suggests the disease's autoimmune nature. Corticosteroid-sparing agents have been used with various degrees of success [1-4]. <h3>Objectives</h3> The aim of our study was to describe our experience treating patients with AIED with immunosuppressive therapy. <h3>Methods</h3> We performed a retrospective chart review of patients diagnosed with AIED at all Mayo Clinic locations for the past 10 years. AIED was diagnosed based on 3 main characteristics: progressive fluctuating hearing loss, response to high-dose corticosteroids, and audiometric evidence supporting sensorineural hearing loss. We recorded audiography results at the time of diagnosis and 1 and 3 months after treatment. We divided treatment efficacy into 2 categories: subjective (patient-reported) and objective (audiometric) improvement. <h3>Results</h3> Thirty-one patients met the inclusion criteria; the mean age was 48.5 years and 17 were women. Patients were initially evaluated by the Department of Otorhinolaryngology and 29 patients were subsequently referred to the Department of Rheumatology with a mean of 12.2 weeks after the first evaluation. While initial hearing loss was more commonly unilateral (21 [67.7%]), it remained unilateral in only 8 (25.8%) patients. Tinnitus, aural fulness, and ear pressure were the 3 most common ear symptoms, followed by imbalance, vertigo, dizziness, and discharge. Treatment with corticosteroids showed improvement of hearing and vestibular symptoms during the first month, but no further improvement by the end of the third month. Other immunosuppressive medications were used with various degrees of response (Table 1). Methotrexate was the second most used therapy with 11 of 17 patients reporting an improvement in symptoms. <h3>Conclusion</h3> Prompt treatment of these patients is vital to prevent irreversible hearing loss. Corticosteroids remain the criterion standard initial treatment; however, our study supports potential use of corticosteroid-sparing agents such as methotrexate for long-term therapy. Our study has some limitations including small patient population and a retrospective design. <h3>References</h3> [1]Das S, Bakshi SS, Seepana R. Demystifying Autoimmune Inner Ear Disease. Eur Arch Otorhinolaryngol 2019;276:3267-3274. https://doi.org/10.1007/s00405-019-05681-5. [2]Mijovic T, Zeitouni A, Colmegna I. Autoimmune Sensorineural Hearing Loss: The Otology-Rheumatology Interface. Rheumatology (Oxford) 2013;52:780-789. https://doi.org/10.1093/rheumatology/ket009. [3]Ciorba A, Corazzi V, Bianchini C, et al. Autoimmune Inner Ear Disease (AIED): A Diagnostic Challenge. Int J Immunopathol Pharmacol 2018;32:2058738418808680. https://doi.org/10.1177/2058738418808680. [4]Loveman DM, de Comarmond C, Cepero R, Baldwin DM. Autoimmune Sensorineural Hearing Loss: Clinical Course and Treatment Outcome. Semin Arthritis Rheum 2004;34:538-543. https://doi.org/10.1016/j.semarthrit.2003.10.002. <h3>Acknowledgements:</h3> NIL. <h3>Disclosure of Interests</h3> None Declared.